Department of Medicine, Jagiellonian University Medical College, Skawińska 8, 31 - 066, Kraków, Poland.
Clin Rev Allergy Immunol. 2013 Feb;44(1):39-50. doi: 10.1007/s12016-011-8266-y.
Churg-Strauss syndrome (CSS) is a rare systemic small-vessel vasculitis that develops in the background of bronchial asthma, which is characterized by eosinophilia and eosinophilic infiltration of various tissues. It belongs to the group of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides. The triggering factors and pathogenesis of CSS are still unknown. The possible role of eotaxin-3 and CCR4-related chemokines in selective recruitment of eosinophils to the target tissues in CSS has been recently suggested, but the role of eosinophilic inflammation in the development of vasculitic lesions is not completely understood. From the clinical view, two distinct phenotypes of the disease are slowly emerging depending on the ANCA-positivity status. Glucocorticoids are still the mainstay of treatment; however, data are accumulating regarding the beneficial role of novel immunosuppressants and biologic compounds, especially in patients with poorer prognosis.
变应性肉芽肿性血管炎（CSS）是一种罕见的系统性小血管血管炎，发生于支气管哮喘背景下，其特征为嗜酸性粒细胞增多和各种组织的嗜酸性粒细胞浸润。它属于抗中性粒细胞胞质抗体（ANCA）相关性血管炎的范畴。CSS 的触发因素和发病机制尚不清楚。最近有人提出，嗜酸性粒细胞趋化因子 eotaxin-3 和 CCR4 相关趋化因子可能在 CSS 中选择性募集嗜酸性粒细胞到靶组织中发挥作用，但嗜酸性粒细胞炎症在血管炎病变发展中的作用尚不完全清楚。从临床角度来看，根据抗中性粒细胞胞质抗体（ANCA）阳性状态，该病正在缓慢出现两种截然不同的表型。糖皮质激素仍然是治疗的主要方法；然而，新型免疫抑制剂和生物制剂有益作用的数据正在不断积累，尤其是在预后较差的患者中。
